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[儿童遗传性心律失常与心脏手术后遗症]

[Genetic cardiac dysrhythmias and the sequelae of heart surgery in children].

作者信息

Popescu V, Dragomir D

出版信息

Rev Pediatr Obstet Ginecol Pediatr. 1989 Oct-Dec;38(4):289-301.

PMID:2518577
Abstract

Genetic cardiac dysrhythmias, although rare in pediatrics, may be often the first manifestation of the main disease or may determine the vital prognosis in some of these diseases (e. g. Friedreich's ataxia and Duchenne's progressive muscular dystrophy). This is more so, as at present, technical possibilities of implanting pacemakers, at ever smaller ages, are ever so great. The first part of the paper deals with these aspects, reviews, and analyses a large variety of genotypic diseases (metabolism genetic diseases, cardio-skeletal syndromes, neuromuscular diseases, cardio-auditory syndromes, etc.) and also a series of tachydysrhythmias or isolated familial conduction disturbances (blocks). Within each entity, the authors describe the main elements allowing the diagnosis, with special references on the rhythm and cardiac conduction disturbances that may appear, and with the necessary therapeutical considerations. The second part of the material presented is devoted to the cardiac dysrhythmias following the surgical intervention on heart, frequently used at present in the congenital heart diseases, at ever smaller ages due to the special progress in anesthesia and intensive care techniques. Correlation of various types of surgeries with the anatomical lesions of the excitation-conduction system they can involve, makes easier the understanding of the nature and appearance risks of some postsurgical rhythm and cardiac conduction disturbances. The distinction is made between early postsurgical dysrhythmias--that are often episodic--and tardy dysrhythmias that may relapse or become chronic, thus raising difficult treatment problems and sometimes require a secondary pacemaker implantation, given the major handicap they represent for the child.

摘要

遗传性心律失常在儿科虽属罕见,但可能常常是主要疾病的首发表现,或者可能决定其中一些疾病的生命预后(例如弗里德赖希共济失调和杜兴氏进行性肌营养不良)。鉴于目前在越来越小的年龄植入起搏器的技术可能性非常大,情况更是如此。本文的第一部分论述了这些方面,回顾并分析了多种基因型疾病(代谢性遗传疾病、心脏骨骼综合征、神经肌肉疾病、心脏听觉综合征等)以及一系列快速性心律失常或孤立性家族性传导障碍(阻滞)。在每个病种中,作者描述了有助于诊断的主要因素,特别提及可能出现的节律和心脏传导障碍,并给出了必要的治疗考量。所呈现材料的第二部分致力于探讨心脏手术后的心律失常,由于麻醉和重症监护技术的特殊进展,目前在先天性心脏病中,越来越小的年龄就经常进行心脏手术。将各种类型的手术与其可能涉及的兴奋传导系统的解剖学病变相关联,有助于更轻松地理解某些术后节律和心脏传导障碍的性质和出现风险。区分了早期术后心律失常(通常为发作性)和晚期心律失常(可能复发或变为慢性),晚期心律失常会引发棘手的治疗问题,有时需要二次植入起搏器,因为它们给患儿带来了严重的不便。

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